Therapeutic Strategies in Children with an Isolated Unilaterally Absent Proximal Pulmonary Artery

• Published in: Pediatric cardiology Vol. 31; no. 5; pp. 607 - 610
• Main Authors: Krammoh, Euloge K., Bigras, Jean-Luc, Prsa, Milan, Lapierre, Chantal, Miró, Joaquim, Dahdah, Nagib S.
• Format: Journal Article
• Language: English
• Published: New York Springer-Verlag 01.07.2010; Springer
• Subjects: Anastomosis, Surgical; Anticoagulants - therapeutic use; Blood Vessel Prosthesis Implantation; Cardiac Catheterization; Cardiac Surgery; Cardiology; Catheterization; Children's furniture; Clopidogrel; Combined Modality Therapy; Congenital heart disease; Echocardiography; Female; Genetic disorders; Heart Defects, Congenital - diagnosis; Heart Defects, Congenital - drug therapy; Heart Defects, Congenital - surgery; Humans; Infant; Infant, Newborn; Life Expectancy; Male; Medicine; Medicine & Public Health; Original Article; Pediatrics; Pulmonary Artery - abnormalities; Quality of Life; Vascular Surgery; Vasodilator Agents; Agenesis; Stent; Pulmonary artery; Childhood
• ISSN: 0172-0643; 1432-1971
• DOI: 10.1007/s00246-010-9689-6

Therapeutic strategies for isolated unilateral absence of a proximal pulmonary artery remain unclear. The natural history of the disease, or thrombosis of primary surgical anastomosis, leads to exclusion of the affected lung with increased risk of intrapulmonary bleeding, impaired quality of life, and shortened life expectancy. We herein describe our two-stage approach in a small series of patients starting with interventional catheterization followed by surgical anastomosis. Other medical interventions, such as anticoagulation and pulmonary vasodilatation, are key factors to successfully restore pulmonary circulation in this rare defect.